• Users Online: 1901
  • Print this page
  • Email this page
REVIEW
Year : 2018  |  Volume : 1  |  Issue : 4  |  Page : 117-124

Pediatric high-grade glioma: A heterogeneous group of neoplasms with different molecular drivers


1 Department of Radiological, Oncological and Pathological Sciences, Sapienza University of Rome, Rome, Italy
2 Department of Experimental Medicine, Sapienza University of Rome, Rome; Laboratory of Molecular Neuropathology, IRCCS Neuromed, Pozzilli, Italy
3 Department of Hematology and Pediatric Hematology-Oncology, Pediatric Oncology Unit, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan, Italy
4 Laboratory of Molecular Neuropathology, IRCCS Neuromed, Pozzilli, Italy
5 Department of Radiological, Oncological and Pathological Sciences, Sapienza University of Rome, Rome; Laboratory of Molecular Neuropathology, IRCCS Neuromed, Pozzilli, Italy

Correspondence Address:
Dr. Felice Giangaspero
Viale Regina Elena 324, 00161, Rome
Italy
Login to access the Email id

Source of Support: None, Conflict of Interest: None


DOI: 10.4103/glioma.glioma_27_18

Rights and Permissions

High-grade gliomas (HGGs) in pediatric age have the same bad prognosis as those arising in adults. Approximately one-half of HGGs in children occur in the brain stem, most frequently within the pons as diffuse intrinsic pontine glioma or other midline structures. Although they have the same histological appearance of adult malignant gliomas, in recent years, the extensive use of molecular profiling techniques has demonstrated significant molecular differences between the two age groups. These data have led to a major reclassification of pediatric HGG (pHGG) based on molecular subgrouping with significant clinical correlations in terms of age at presentation, anatomical location, and prognosis. The most important molecular groups are: (1) the histone mutations related pHGG, that is, H3.K27-mutated midline and H3.G34-mutated hemispheric pHGG; (2) the rare isocitrate dehydrogenase (IDH)-mutated pHGG occurring mainly in adolescents; and (3) the H3-/IDH wild type, a heterogenous group of pHGG still object of further molecular stratification. Another important group of pHGG is that occurring in patients with cancer predisposition syndromes such as Li-Fraumeni syndrome, constitutional mismatch repair deficiency, and neurofibromatosis-1 (NF1). In this review, the different subgroups of pHGG and their major driver molecular alterations will be discussed.


[FULL TEXT] [PDF]*
Print this article     Email this article
 Next article
 Previous article
 Table of Contents

 Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
 Related articles
 Citation Manager
 Access Statistics
 Reader Comments
 Email Alert *
 Add to My List *
 * Requires registration (Free)
 

 Article Access Statistics
    Viewed9399    
    Printed265    
    Emailed2    
    PDF Downloaded1155    
    Comments [Add]    
    Cited by others 5    

Recommend this journal